CEREBRO-HEPATO-RENAL SYNDROME. REPORT OF A CASE WITH HISTOCHEMICAL AND ULTRASTRUCTURAL OBSERVATIONS

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Abstract
A new case of the cerebro-hepato-renal syndrome is being reported. The neuropathologic changes consisted of developmental abnormalities of cere-bral and cerebellar cortex and of the inferior olives. In addition, there were metabolic changes in the central nervous system, indicated by an accumula-tion of lipid within histiocytes or free in gray and white matter, deficiency in myelination and gliesis of the white matter, and marked proliferation of protoplasmic astrocytes in the gray matter. A unique feature of this case was the presence of numerous clusters of lipid-filled macrophages and occasional cholesterol crystals in the molecular layer of the cerebellum. The histochemi-cal reactions and ultrastructural appearances of the lipid deposits are sugges-tive of accumulation of several different types of lipids.